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PIGV Antibody

  • 中文名稱：
    PIGV兔多克隆抗體
  • 貨號：
    CSB-PA868321LA01HU
  • 規(guī)格：
    ￥440
  • 促銷：
    小規(guī)格抗體限時一口價
  • 圖片：
    • Western Blot
      Positive WB detected in: A549 whole cell lysate
      All lanes: PIGV antibody at 4.6µg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 56 kDa
      Observed band size: 56 kDa
    • IHC image of CSB-PA868321LA01HU diluted at 1:200 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • Immunofluorescence staining of Hela cells with CSB-PA868321LA01HU at 1:66, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) PIGV Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    PIGV
  • 別名：
    GPI mannosyltransferase 2 antibody; GPI mannosyltransferase II antibody; GPI MT II antibody; GPI-MT-II antibody; Phosphatidylinositol glycan biosynthesis class V protein antibody; Phosphatidylinositol-glycan biosynthesis class V protein antibody; PIG-V antibody; Pigv antibody; PIGV_HUMAN antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human GPI mannosyltransferase 2 protein (400-469AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated

    本頁面中的產(chǎn)品，PIGV Antibody (CSB-PA868321LA01HU)，的標記方式是Non-conjugated。對于PIGV Antibody，我們還提供其他標記。見下表：

    可提供標記
    標記方式 貨號 產(chǎn)品名稱 應(yīng)用
    HRP CSB-PA868321LB01HU PIGV Antibody, HRP conjugated ELISA
    FITC CSB-PA868321LC01HU PIGV Antibody, FITC conjugated
    Biotin CSB-PA868321LD01HU PIGV Antibody, Biotin conjugated ELISA
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA, WB, IHC, IF
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:200-1:500
    IF 1:50-1:200
  • Protocols：
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

產(chǎn)品評價

靶點詳情

  • 功能：
    Alpha-1,6-mannosyltransferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers the second mannose to the glycosylphosphatidylinositol during GPI precursor assembly.
  • 基因功能參考文獻：
    1. Data indicate that mannosyltransferases PIGV mutations are the major cause of hyperphosphatasia-mental retardation syndrome (HPMRS) which displays a broad clinical variability regarding associated malformations and growth patterns. PMID: 24129430
    2. PIGV is the rate-limiting enzyme in GPI biosynthesis under limited dolicholphosphate mannose availability. PMID: 23694781
    3. Hyperphosphatasia resulted from secretion of ALP, a GPI-anchored protein normally expressed on the cell surface, into serum due to PIGV deficiency. PMID: 22228761
    4. novel compound heterozygous mutations in the PIGV gene c.467G>A and c.1022C>A and a homozygous mutation c.1022C>A in hyperphosphatasia-mental retardation syndrome PMID: 21739589
    5. PIGV mutations are associated with hyperphosphatasia mental retardation syndrome. PMID: 20802478
    6. PIG-V is the second mannosyltransferase in GPI anchor biosynthesis. PMID: 15623507

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  • 相關(guān)疾病：
    Hyperphosphatasia with mental retardation syndrome 1 (HPMRS1)
  • 亞細胞定位：
    Endoplasmic reticulum membrane; Multi-pass membrane protein.
  • 蛋白家族：
    PIGV family
  • 數(shù)據(jù)庫鏈接：

    HGNC: 26031

    OMIM: 239300

    KEGG: hsa:55650

    STRING: 9606.ENSP00000078527

    UniGene: Hs.259605